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. 2024 Jun 22:20:123-131.
doi: 10.1016/j.xjon.2024.06.007. eCollection 2024 Aug.

Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease

Christina L Greene  1 , Antonia Schulz  1 , Mariana Chávez  1 , Steven J Staffa  2 , David Zurakowski  2 , Kevin G Friedman  3 , Sitaram M Emani  1 , Christopher W Baird  1
Affiliations

Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease

Christina L Greene et al. JTCVS Open. .

Abstract

Objective: To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease.

Methods: An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared.

Results: Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (P < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; P = .004).

Conclusions: Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.

Keywords: congenital heart disease; trisomy 13; trisomy 18.

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Conflict of interest statement

John David Korngiebel Trust Fund as a source of funding for this article. The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Figures

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Graphical abstract
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Survival in patients undergoing operation was significantly higher than those who did not.
Figure 1
Figure 1
Referrals to our center from 1985 until October 2023. Sixty-two patients were referred with 34 undergoing operative and 28 undergoing nonoperative treatment. There has been a steady increase in overall referrals and a majority of patients from the operated group have been treated during the past 5 years (21 out of 34 patients).
Figure 2
Figure 2
There was no difference in Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category distribution between the 34 operated and 28 nonoperated patients (P = .63).
Figure 3
Figure 3
Survival for operated patients was significantly higher than nonoperated patients throughout the study period (P < .001). Shading indicates 70% CI.
Figure 4
Figure 4
A, Survival of operated trisomy (T) 13 and 18 patients versus nonoperated. Shading indicates 70% CI. B, Survival for operated T18 patients was significantly higher than nonoperated T18 patients (P = .006). Patients with T13 had worse survival compared with patients with T18. Survival for patients with T13 undergoing operation was better than nonoperated patients with T13 but was limited by small sample size and was not statistically significant (P = .06).
Figure 5
Figure 5
Patients with trisomy 13 and trisomy 18 undergoing operation with congenital heart disease had improved survival compared with nonoperated patients.
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